1. [Successful immunosuppressive therapy in female hemophilia A developing inhibitor after perioperative administration of factor VIII products].
- Author
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Yamaguchi M, Takaki Y, Yamasaki Y, Oya S, Nakamura T, Morishige S, Aoyama K, Mouri F, Takase R, Matsuo Y, Osaki K, Nagafuji K, and Okamura T
- Subjects
- Female, Humans, Middle Aged, Factor VIII therapeutic use, Hemarthrosis, Immunosuppression Therapy, Hemophilia A drug therapy, Hemostatics therapeutic use
- Abstract
A 62-year-old woman was diagnosed as a hemophilia A carrier (factor VIII activity 35%) on preoperative examination of an ovarian tumor. A total of 35,600 units of recombinant factor VIII products was administered perioperatively. On postoperative day 95, a subcutaneous hematoma formed and immunosuppressive therapy with prednisolone was started based on an APTT of 66 seconds, factor VIII (FVIII) activity of 3%, and FVIII inhibitor of 1 BU/ml. During this treatment, the patient was hospitalized due to ankle joint bleeds and required hemostatic treatment, but the inhibitor disappeared and FVIII activity recovered to 30% after postoperative day 438 with cyclophosphamide. F8 analysis revealed the patient carried a heterozygosity of p.Arg391Cys, which has previously been categorized as cross-reacting material (CRM)-positive severe hemophilia A. No high-risk mutations for inhibitor development were found. We also report the results of a desmopressin acetate hydrate test administered to the patient to prepare for future treatment in case of hemorrhage, since high-dose FVIII administration may have been a factor in inhibitor development.
- Published
- 2024
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